ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (5)
Malignant peripheral nerve sheath tumor
Print
Other Resources UpToDate PubMed

Malignant peripheral nerve sheath tumor

Print Images (5)
Contributors: Andrea Wasilewski MD, Christine Osborne MD, Jamie Adams MD, Benjamin L. Mazer MD, MBA, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Malignant peripheral nerve sheath tumors (MPNSTs), formerly known as neurofibrosarcomas, are tumors that grow around peripheral nerves. They account for less than 10% of peripheral nerve tumors. Patients present with a painful mass along a nerve distribution. Sensory and motor deficits of the nerve are common. MPNSTs tend to occur on the extremities and trunk and along major nerve trunks such as the brachial plexus.

Neurofibromas may transform into MPNSTs. This is of specific concern in patients with neurofibromatosis, who may have a high number of peripheral nerve tumors.

MPNSTs have a poor prognosis generally, with a worse prognosis in people with large tumors or neurofibromatosis. The primary treatment is surgical resection.

Codes

ICD10CM:
C47.9 – Malignant neoplasm of peripheral nerves and autonomic nervous system, unspecified

SNOMEDCT:
404037002 – Malignant peripheral nerve sheath tumor

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 04/05/2019
Last Updated: 04/23/2019
Copyright © 2019 VisualDx®. All rights reserved.
Malignant peripheral nerve sheath tumor
Print 5 Images
View all Images (5)
(with subscription)
Malignant peripheral nerve sheath tumor : Numbness, Paresthesias
Copyright © 2019 VisualDx®. All rights reserved.